Malignant Thyroid-Type Papillary Neoplasm in Struma Ovarii: a Case Report
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Open Access Case Report DOI: 10.7759/cureus.6450 Malignant Thyroid-type Papillary Neoplasm in Struma Ovarii: A Case Report Syed Adeel Hassan 1 , Ali Akhtar 2 , Noor Ul Falah 3 , Fahad N. Sheikh 4 1. Internal Medicine, Dow University of Health Sciences, Karachi, PAK 2. Internal Medicine, Shaukat Khanum Memorial Cancer Hospital and Research Centre, Lahore, PAK 3. Pathology, Shaukat Khanum Memorial Cancer Hospital and Research Centre, Lahore, PAK 4. Internal Medicine, Sahiwal Medical College, Sahiwal, PAK Corresponding author: Syed Adeel Hassan, [email protected] Abstract Papillary thyroid carcinoma (PTC) arising in a coexistent struma ovarii (SO) is a rare malignancy. It manifests with abdominal symptoms such as palpable mass, pain, distension, and possibly ascites. It is usually diagnosed postoperatively, and its histopathological diagnostic criteria remain identical to that of papillary carcinoma of the thyroid gland. Due to the relative rarity of the disease, definitive guidelines for its overall management are still undefined. We present a case of a 51-year old female with suspicion of a left ovarian tumor due to her presenting symptoms: raised serum CA-125 levels and abnormal abdominopelvic CT scan findings. She underwent complete surgical debulking of the mass (total abdominal hysterectomy (TAH), bilateral salpingo-oophorectomy (BSO), omentectomy, appendectomy, and pelvic lymphadenectomy). The mass was postoperatively diagnosed by histopathology as PTC in SO (stage IA). Furthermore, our patient did not receive any adjuvant treatment. The patient has been disease-free for 24 months post-surgery and is scheduled for regular biannual follow-ups. Categories: Internal Medicine, Pathology, Oncology Keywords: papillary thyroid carcinoma, struma ovarii, thyroid gland, ovarian tumor, surgical debulking, psammoma body, dystrophic calcification, orphan annie eye nuclei, ca-125, stained glass appearance Introduction Struma ovarii (SO) is defined as the presence of a single predominant type of mature thyroid tissue in a coexisting ovarian tumor. To date, only about 200 cases have been reported in the literature. It presents more frequently between the ages of 40 and 60 [1]. The majority of SO are benign and belong to the category of mature teratomas, which account for 2-5% of overall benign mature teratomas [2,3]. Rarely, a malignant type of tissue may arise in the background of SO, thereby transforming it into a thyroid-type carcinoma. In such malignant cases, the most common histological subtypes, in the order of decreasing prevalence, include papillary thyroid carcinoma (PTC), follicular thyroid carcinoma, and highly differentiated follicular carcinoma (HDFCO) [3]. Malignant struma ovarii (MSO) are usually asymptomatic. However, thyrotoxicosis is reported to be present in 5-8% of the affected patients [4]. Our case discussion pertains to the rare variant Received 11/25/2019 Review began 12/19/2019 of MSO harboring a focus of the classical or conventional type of PTC. Due to its rarity, sufficient data are Review ended 12/19/2019 not available regarding an approach to diagnosis, treatment, and prognosis. Therefore, our report discusses Published 12/23/2019 its clinicopathological features, diagnostic criteria, and management strategies. © Copyright 2019 Hassan et al. This is an open access article distributed under the terms of the Case Presentation Creative Commons Attribution License A 51-year-old female (gravida 6, para 6) presented to our walk-in clinic with a four-month history of CC-BY 3.0., which permits unrestricted progressive lower abdominal pain and distention in the lower left quadrant. She complained of indigestion use, distribution, and reproduction in any medium, provided the original author and and had had significant weight loss in the last two months. Her past medical history revealed she had been source are credited. operated for cholelithiasis 14 years ago. The patient's family history was insignificant for other comorbidities and cancer. Physical examination was consistent with a palpable abdominopelvic mass (8-10 weeks in size) on the lower left side. A CT scan of the abdomen and pelvis yielded a large left adnexal mass measuring approximately 8.1 x 6.7 cm (Figure 1). The architecture of the mass depicted compositions of both solid and cystic components (Figure 2). The uterus and the right adnexa showed no signs of disease involvement and therefore were deemed to be normal. However, small-to-moderate volume ascitic fluid with peritoneal stranding was noted. No pelvic invasion, lymphadenopathy, or secondary implants were identified. Digital chest X-ray and CT of the chest to rule out systemic spread showed clear lung fields and no pleural involvement. Our patient also tested positive for serum CA-125, whose levels were noted at 413 units/mL (normal range: 0-35 units/mL). The results of other diagnostic tests such as thyroid profile, serum LDH, alpha-fetoprotein, and beta-HCG were within normal limits. Therefore, based on imaging results and serum CA-125 levels, the initial diagnosis of the primary ovarian tumor was made. Subsequently, our patient underwent total abdominal hysterectomy (TAH) with bilateral salpingo-oophorectomy (BSO). Omentectomy, appendectomy, pelvic lymphadenectomy, and peritoneal biopsy were also performed. On gross examination of the surgically resected specimen, a multiloculated solid and cystic left ovarian mass was noted. Uterus, right ovary, and How to cite this article Hassan S, Akhtar A, Falah N, et al. (December 23, 2019) Malignant Thyroid-type Papillary Neoplasm in Struma Ovarii: A Case Report . Cureus 11(12): e6450. DOI 10.7759/cureus.6450 bilateral fallopian tubes showed no gross signs of disease involvement. Adhesions noted between the small bowel and urinary bladder wall were removed via adhesiolysis. The postoperative recovery was uneventful and satisfactory. FIGURE 1: Sagittal CT scan of the abdomen/pelvis Sagittal CT depicting a large left adnexal mass (red circle) 2019 Hassan et al. Cureus 11(12): e6450. DOI 10.7759/cureus.6450 2 of 8 FIGURE 2: Coronal CT scan of the abdomen/pelvis A left multiloculated adnexal mass (blue circle), with solid and cystic components, is appreciated. The differential signal intensity between locules imparts a stained glass appearance. Histopathological studies of the left ovarian mass revealed a focus of classical PTC measuring 2 cm in the background of a SO (Figures 3-5). The focus of carcinoma lacked features of surface involvement or capsular rupture. Right adnexa, left fallopian tube, endometrium, appendix, omentum, pelvic lymph nodes, and peritoneum showed no signs of malignant involvement. Therefore, the tumor was limited to one ovary with its capsule intact and showed no signs of involvement on the ovarian surface. As per the International Federation of Gynecology and Obstetrics (FIGO) system for ovarian cancer staging, our specimen was considered IA (pT1aN0M0). Immunohistochemistry was not pursued owing to the histological confirmation of the tumor. Due to a lack of metastasis and localization of the tumor in the left ovary, it was decided that the patient should not be given any form of adjuvant therapy. Postoperatively, the patient was surveilled for thyroid carcinoma with the help of thyroid panel tests and thyroid gland ultrasonography (done twice). The results of the surveillance were normal. FIGURE 3: Low-resolution image depicting a focus of PTC in a struma ovarii PTC: papillary thyroid carcinoma Multiple complex and branched papillae with central fibrovascular cores are seen, with presumably dystrophic calcification (psammoma body) in one papilla. The identification of psammoma bodies in a thyroid-type papillary neoplasm is highly supportive of malignancy. However, they are better visualized on a higher resolution 2019 Hassan et al. Cureus 11(12): e6450. DOI 10.7759/cureus.6450 3 of 8 FIGURE 4: High-resolution image depicting the papillary architecture of the tumor The papillary architecture of the tumor is evident with stromal tissue in the fibrovascular cores. Papillae are lined by cuboidal to low columnar neoplastic cells with characteristic ‘Orphan-Annie eye’ nuclei, having clear ground-glass chromatin (black arrow). Nuclear overcrowding is evident (green arrow). Some nuclei show coffee-bean appearance, with longitudinal grooves due to nuclear membrane infoldings (yellow arrow) FIGURE 5: Low-resolution image showing mature teratoma on the left side of the image adjacent to the focus of papillary thyroid carcinoma arising in a struma ovarii 2019 Hassan et al. Cureus 11(12): e6450. DOI 10.7759/cureus.6450 4 of 8 PTC: papillary thyroid carcinoma The major bulk of the PTC can be seen on the right side. The benign teratomatous tissue shows dense stroma with multiple, relatively well-circumscribed sebaceous glands (black arrows) and overlying stratified epithelium. Benign-looking thyroid follicular tissue with eosinophilic colloid and flattened epithelial lining can also be seen at the top right corner Our patient was scheduled for biannual follow-ups and is now disease-free two years post-resection. Postoperative serial measurements of thyroglobulin, anti-thyroglobulin antibody, serum CA-125 levels and contrast CT imaging of chest, abdomen, and pelvis have been conducted. CT has not shown any residual or recurrent disease so far. The patient is euthyroid with normal postoperative thyroglobulin and anti- thyroglobulin antibody levels. Serum CA-125 levels have shown an initial decreasing trend post-surgery. However, during the second follow-up visit, a raised serum CA-125 level was noted, which regressed to baseline during the subsequent follow-ups. The preoperative and postoperative progression patterns of serum CA-125 levels are noted below in Table 1. Patient’s stage Serum CA-125 level (U/mL) Preoperative 413 One month post-surgery 26.7 First follow-up 18.3 Second follow-up 38.3 Third follow-up 18 Fourth follow-up 14.7 TABLE 1: Postoperative follow-up of patient’s serum CA-125 levels CA 125: cancer antigen 125; normal serum CA 125: <35 U/mL Discussion Germ cell tumors of the ovary are known to arise from any form of pathogenic insult to the normal ovarian germ cell lineage.